Angiosarcoma of the adrenal gland is an extremely rare malignancy. We report a case of a 59-year-old female who presented with abdominal pain and profound anemia. A 7 cm enhancing, lipid-poor adrenal mass with calcifications that extended posterior to the vena cava was identified on imaging. Patient underwent right adrenalectomy with retroperitoneal lymph node dissection. Pathology demonstrated angiosarcoma of the adrenal gland. Consolidative treatment included adjuvant radiation and chemotherapy. Patient remains disease free 1...

Olaparib is the first oral poly(ADP-ribose) polymerase inhibitor to be approved as maintenance monotherapy for treatment of patients with platinum-sensitive relapsed BRCA-mutated (BRCAm) serous ovarian cancer. This review provides practical guidance on the use of olaparib (capsule formulation) in the maintenance setting. The article focuses on the key toxicities that can arise with olaparib therapy and recommendations for their management. Nausea, vomiting, fatigue and anemia are the most commonly reported adverse events in olaparib clinical trials and are generally mild to moderate and transient in nature in most patients...

BACKGROUND: Acquired Factor (F)XIII deficiency is a very rare bleeding diathesis with a potentially fatal outcome, previously described in the context of autoimmune disorders and leukemias. There is minimal information on autoantibody characterization and the role of antifibrinolytic therapy in patient management. CASE REPORT: A 79-year-old woman with a 3-month history of bruising and heavy menorrhagia presented with ongoing vaginal bleeding, symptomatic anemia, and a right thigh hematoma...

BACKGROUND: Anal stricture is a disabling condition which is often unresponsive to conservative medical management. The complications of surgical procedures such as dilatations and anoplasty make it a formidable treatment challenge. Through this case, we report and explore a new medical treatment for ano-rectal strictures with an analogy to Plummer Vinson syndrome. A 69-year-old male presented with chronic constipation, rectal pain, and easy fatigability. The physical exam was negative for anal fissure and a digital rectal examination could not be completed because an index finger could not be advanced through the narrowed anus...

Autoimmune hemolytic anemia (AIHA) is a rare and heterogeneous disease that affects 1 to 3/100 000 patients per year. AIHA caused by warm autoantibodies (w-AIHA), ie, antibodies that react with their antigens on the red blood cell optimally at 37°C, is the most common type, comprising ∼70% to 80% of all adult cases and ∼50% of pediatric cases. About half of the w-AIHA cases are called primary because no specific etiology can be found, whereas the rest are secondary to other recognizable underlying disorders...

Since 1998, the National Institutes of Health has funded 5 randomized controlled trials (RCTs) for primary and secondary prevention of strokes in children with sickle cell anemia (SCA). In a systematic fashion, these trials have significantly advanced the care of children with SCA. In the absence of an RCT, clinicians are often compelled to make decisions at the bedside, based on experience, observational studies, and principles of hematology. We will provide an initial example that describes how a team-based, learning collaborative developed a multisite standard care protocol with a low budget (<$10 000 per year) to overcome the intrinsic limitations of advancing the care of neurologic complications in sickle cell disease (SCD)...

Myelodysplastic syndrome (MDS) and myeloproliferative disorders are rare in children; they are divided into low-grade MDS (refractory cytopenia of childhood [RCC]), advanced MDS (refractory anemia with excess blasts in transformation), and juvenile myelomonocytic leukemia (JMML), each with different characteristics and management strategies. Underlying genetic predisposition is recognized in an increasing number of patients. Germ line GATA2 mutation is found in 70% of adolescents with MDS and monosomy 7. It is challenging to distinguish RCC from aplastic anemia, inherited bone marrow failure, and reactive conditions...

Heavy menstrual bleeding (HMB), which is the preferred term for menorrhagia, affects ∼90% of women with an underlying bleeding disorder and ∼70% of women on anticoagulation. HMB can be predicted on the basis of clots of ≥1 inch diameter, low ferritin, and "flooding" (a change of pad or tampon more frequently than hourly). The goal of the work-up is to determine whether there is a uterine/endometrial cause, a disorder of ovulation, or a disorder of coagulation. HMB manifest by flooding and/or prolonged menses, or HMB accompanied by a personal or family history of bleeding is very suggestive of a bleeding disorder and should prompt a referral to a hematologist...

Once suspected, the diagnosis of paroxysmal nocturnal hemoglobinuria (PNH) is straightforward when flow cytometric analysis of the peripheral blood reveals a population of glycosyl phosphatidylinositol anchor protein-deficient cells. But PNH is clinically heterogeneous, with some patients having a disease process characterized by florid intravascular, complement-mediated hemolysis, whereas in others, bone marrow failure dominates the clinical picture with modest or even no evidence of hemolysis observed. The clinical heterogeneity is due to the close, though incompletely understood, relationship between PNH and immune-mediated bone marrow failure, and that PNH is an acquired, nonmalignant clonal disease of the hematopoietic stem cells...

Mutations in SUCLA2 result in succinyl-CoA ligase (ATP-forming) or succinyl-CoA synthetase (ADP-forming) (A-SCS) deficiency, a mitochondrial tricarboxylic acid cycle disorder. The phenotype associated with this gene defect is largely encephalomyopathy. We describe two siblings compound heterozygous for SUCLA2 mutations, c.985A>G (p.M329V) and c.920C>T (p.A307V), with parents confirmed as carriers of each mutation. We developed a new LC-MS/MS based enzyme assay to demonstrate the decreased SCS activity in the siblings with this unique genotype...

OBJECTIVE: Eribulin, a microtubule dynamics inhibitor, is approved for the treatment of patients with breast cancer and soft tissue sarcoma. We investigated the efficacy and safety of eribulin in Japanese patients with soft tissue sarcoma. METHODS: This open-label, multicenter, nonrandomized, Phase 2 study enrolled Japanese patients with measurable, advanced/metastatic soft tissue sarcoma of high/intermediate grade and ≥1 prior chemotherapy for advanced disease...

Gastrointestinal angiodysplasia (GIAD) are red flat arborized lesions that are found throughout the entire gastrointestinal tract. GIAD can vary in size and have a range of presentation from occult to life-threatening bleeding. The typical presentation is intermittent bleeding in the setting of iron deficiency anemia. Endoscopy is the primary means of diagnosis and endoscopic therapy is noted to be initially effective. However, rebleeding can be as high as 40% to 50% in patients with small bowel GIAD. This review describes the pathophysiology for the development of GIAD and the current roles of endoscopic, medical, and surgical therapy in its treatment...

Hemolytic uremic syndrome (HUS) is defined by the simultaneous occurrence of hemolytic anemia, thrombocytopenia, and acute kidney injury due to thrombotic microangiopathy (TMA) mainly occurring in renal and cerebral microvessels. Although the most common cause of HUS in children is Shiga toxin-producing Escherichia coli, atypical forms in which Shiga toxin is not the trigger may occur. Research over the last few years has shown that complement dysregulation secondary to mutations of genes coding for proteins involved in the regulation of the alternative pathway of complement account for most forms of atypical HUS (aHUS)...

STUDY OBJECTIVE: To evaluate whether ovulatory dysfunction due to polycystic ovary syndrome (PCOS) is a common underlying etiology of abnormal uterine bleeding (AUB) in adolescents requiring hospitalization and to explore etiology, treatment and complications of AUB with severe anemia in adolescents. DESIGN: We identified females aged 8 to 20 years admitted to a children's hospital for treatment of AUB from January 2000 to December 2014. Our hospital protocol advises hormonal testing for PCOS and other disorders prior to treatment for AUB...

Objective: To assess the incidence, risk factors, and outcomes of perioperative stroke in aged patients undergoing nonneurologic and noncardiovascular surgery. Methods: A total of 21 419 in-patients who were older than 65 years undergoing nonneurologic and noncardiovascular surgery in Peking University People's Hospital from January 2010 to May 2016 were retrospectively recruited in this study.The patients were divided into two groups: patients with perioperative stroke were allocated in stroke group, and the other patients were in the non-stroke group...

The autoimmune cytopenias are a related group of disorders in which differentiated hematopoietic cells are destroyed by the immune system. Single lineage disease is characterized by the production of autoantibodies against red cells (autoimmune hemolytic anemia [AIHA]), platelets (autoimmune thrombocytopenia [ITP]) and neutrophils (autoimmune neutropenia [AIN]) whereas multilineage disease may include various combinations of these conditions. Central to the genesis of this disease is the breakdown of central and/or peripheral tolerance, and the subsequent production of autoantibodies by both tissue and circulating self-reactive B lymphocytes with support from T helper lymphocytes...

The anemia of chronic kidney disease (CKD) is a common comorbidity seen in kidney diseases. It is also associated with increased cardiovascular morbidity and mortality and diminished quality of life. Often, patients with CKD of different stages require erythropoiesis-stimulating agents (ESAs) to maintain their hemoglobin (Hb) within the target range. Darbepoetin alfa is a newer ESA with a longer half-life than recombinant human erythropoietin (EPO). The objective of this study is to assess the efficacy and safety profile of twice-monthly (Q2W) and once a month (1QM) darbepoetin alfa in CKD patients, not on dialysis...

PURPOSE OF REVIEW: Patient blood management aims to improve patient outcome and safety by reducing the number of unnecessary red blood cell transfusions and vitalizing patient-specific anemia reserves. While this is increasingly recognized as best clinical practice in elective surgery, the implementation in the setting of trauma is restrained because of typically nonelective (emergency) surgery and, in specific circumstances, allogeneic blood transfusions as life-saving therapy. RECENT FINDINGS: Viscoelastic diagnostics allow a precise identification of trauma-induced coagulopathy...

OBJECTIVE: Peripartum cardiomyopathy is a rare type of heart failure to which only pregnant women are susceptible. In Puerto Rico there is a paucity of information regarding this condition. In this report we describe our experience with peripartum cardiomyopathy. METHODS: This was a descriptive retrospective study. We conducted a chart review of all the patients with peripartum cardiomyopathy managed at our institution from January 2006 through December 2012. A total of 12 patients qualified for our analysis...

The classical definition of Chronic Obstructive Pulmonary Disease (COPD) as a lung condition characterized by irreversible airway obstruction is outdated. The systemic involvement in patients with COPD, as well as the interactions between COPD and its comorbidities, justify the description of chronic systemic inflammatory syndrome. The pathogenesis of COPD is closely linked with aging, as well as with cardiovascular, endocrine, musculoskeletal, renal, and gastrointestinal pathologies, decreasing the quality of life of patients with COPD and, furthermore, complicating the management of the disease...